Paediatrics Flashcards

1
Q

CCCC

Punched out lytic skull lesions with bevelled edges?

A

Eosinophilic granuloma

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2
Q

Ground glass appearance lytic skull lesion ?

A

Fibrous dysplasia

Addition: Cortical expansion and well-defined sclerotic margins

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3
Q

Well-defined lucencies paired in the posterior para-sagittal location?

A

Parietal Foramina

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4
Q

Monoventricle, thalami and basal ganglia are fused?

A

Alobar Holoprosencephaly

Addition: Most severe form.

**No falx or corpus callosum

Complete absence of cleavage with “pancake” of anterior cerebral tissue, crescent-shaped anterior monoventricle communicating with large dorsal cyst,** fused thalami**

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5
Q

Absent septum pellucidum ?

A

Lobar Holoprosencephaly

Mildest from of holoprosencephaly

Cleavage is apparently back to front (opposite the corpus callosum ) so posterior fossa is normal in mildest form

Interhemispheric fissure & falx are mostly formed with partial nonseparation of frontal lobes

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6
Q

Partially formed falx, rudimentary third ventricle, fused at the thalamus. posterior brain is normal?

A

Semilobar Holoprosencephaly

Absent frontal lobe cleavage with parietooccipital lobe separation

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7
Q

Soft tissue neck lump anterior border of SCM and posterior to the submandibular gland?

A

2nd Branchial cleft cyst

Nb- can extend between the carotid bifurcation but doesn’t splay the bifurcation ie carotid body tumour (Chemoductoma) (age group 50-60s)

DDx Necrotic level 2 node
-older child/adult
-Thyroid cancer or HPV related nasopharyngeal

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8
Q

Soft tissue lump at the ‘peri-parotid’ region?

A

1st Branchial cleft cyst

Cystic mass near pinna & EAC or extending from EAC to angle of mandible

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9
Q

Soft tissue lump at the base of the SCM?

A

3rd Branchial cleft cyst

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10
Q

Multilocular fluid filled mass with internal septations at the the posterior triangle?

A

Cystic hygroma

Nb - Associated with turners and hydrops/downs

T2 bright. Doesnt enhance. No internal flow.

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11
Q

Difference and similarities between Epidermoid cyst and arachnoid cyst

A

Both - CSF density on CT, can occupy CPA, homogeneous.

Epidermoid - Restricts diffusion. Hyperintensity on FLAIR

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12
Q

Complete opacification of the maxillary sinus, peripheral enhancemen, expanded sinus with smooth remodeling of walls?

A

Mucocoele

Nb - Ostium of given sinus is occluded thus complete opacification.
*- Doesn’t extend beyond the cavity. *
- **Expands cavity and can cause bone thinning. **

Associated with* CF and prior trauma*

Peripheral enhancment allows differentiate from neoplasm

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13
Q

Low attenuation mass in the sinus that expands the cavity and extends into the meatus ?

A

Antrochonal polyp/Solitary Sinonasal Polyp

*Nb - T2 bright and also peripheral enhancement. *

Typically doesn’t completely opacify the sinus

Solitary ‘dumbell shaped-‘ polypoid mass fills maxillary antrum, then spills through enlarged maxillary ostium and infundibulum or accessory ostium into nasal cavity

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14
Q

Seizures, atrophy of hippocampus and high T2/FLAIR signal?

A

Mesial temporal sclerosis

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15
Q

Difference between Limbic encephalitis and HSV in paediatric setting?

A

Limbic-
Paraneoplastic (bronchial small cell) or autoimmune.
Psychotic features.
No haemorrhage on imaging

HSV -
Can have haemorrhage. Cytotoxic oedema and mass effect.
- HSV -1 . (Adults HSV-2)
- DWI restriction
- Bilateral but asymmetric with late hemorrhage
- Basal ganglia usually spared

Sperate it from Japanese encephalitis = doesnt spare the basal ganglia/thalamus.

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16
Q

Intraventricular lobulated mass in trigone of lateral ventricle. Smooth, large and small foci of calcification on CT. Lateral ventricle is dilated?

A

Choroid plexus papilloma

Hyperdense on CT.
Iso on T1 and hyperintense on T2.
**Intense homogenous enhancement. **

Nb in adults - occur predominantly in 4th ventricle

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17
Q

Mass in the inferior 4th ventricle of child with hypointensity on T1 and foci of high T2 signal ?

A

Ependymoma

4th ventricle mass (± indistinct interface with floor of 4th ventricle)
Fine, stippled Ca⁺⁺ common (50%)
± cysts, hemorrhage
Hydrocephalus common

Hetergenous Signal as Ca2+/Blood products
- overall T1 iso/hypointense and T2 hyerpintense

Anterolateral extension through recess(es) into CPA cistern
Posteroinferior extension through foramen of Magendie into cisterna magna

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18
Q

Enlargement of the SCM in the neonatal period ?

A

Fibromatosis coli

‘Two heads of the SCM’

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19
Q

Erosive soft tissue mass in the middle ear ?

A

Cholesteatoma

Nb - its termed ‘Congenital’ if less than < 5yrs.

Isointense T1 and hyperintense T2

Congenital cholesteatoma are less destructive.

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20
Q

Non- erosive soft tissue mass in the middle ear ?

A

Choleterol granuloma

Nb - Cystic strucutres with Blood and cholesterol =** High T1 and T2.**

If located in petrous apex can be aggressive

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21
Q

Most common cause of premature suture closure?

A

Sagittal
Scaphocephaly - ‘scapho’ latin for boat - LONG and NARROW

Trigonocephaly = **POINTED forehead = closure of metopic **suture, eyes close together

Brachycephaly - Coronal. = SHORT and WIDENDED
Unilateral more common. Harlequins eye

Turricephaly (bilateral) or Plagiocepahly (unilateral) - Lambdoid

Cloverleaf - all

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22
Q

Early closure of which suture causes elevation of the superolateral corner of the orbit?

A

Coronal

Harlequin eye / Brachycephaly

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23
Q

What brain tumour is found in Tuberous Sclerosis?

A

Subependymal giant cell astrocytoma (SEGA)

aka Intraventricular astrocytoma of tuberous sclerosis complex (TSC

Enlarging, enhancing foramen of Monro mass in patient with TSC

Tuberous sclerosis is triad of
-facial angiofibromas
-seizures
-mental retardation

Cortical/subcortical TUBERS
- expand overlying gyri, low on CT,
- can have cystic and Ca2+ transformation.

DDX - TORCH namely toxo and CMV can cause periventricular Ca2+

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24
Q

What are the GU/GI manifestations of Tuberous sclerosis?

A

Renal cysts,
Renal AMLs
splenic adenoma
Hamartomatous rectal polyps

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25
Q

What are the GI/GU manifestations of VHL?

A

RCC
Renal and pancreatic cysts
Phaeochromocytoma

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26
Q

What are the GI/GU manifestations of NF-1

A

Hirschsprungs
Carcinoid
Renal artery stenosis

Nb none with NF-2

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27
Q

Highly vascular mass in nasopharynx eroding the medial ptyergoid plates and arising from sphenopalatine fossa?

A

Juvenile angiofibroma

Supplied by the internal maxillary artery. Embolise it.

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28
Q

Pineal mass in boy with precocious puberty?

A

Germinoma

Pineal mass with Central localized Ca⁺⁺ & bithalamic extension

Nb Germinoma and Pineoblastoma difficult to differentiate on imaging.

Can be Suprasellar mass with diabetes insipidus (DI)

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29
Q

Child with large, heterogeneous hyperdense pineal mass with peripheral Ca⁺⁺

A

Pineoblastoma

**Scattered (“exploded”) Ca⁺⁺ rather **than localized (“engulfed”) Ca⁺⁺ in germinoma

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30
Q

Differentiate a medulloblastoma from a pilocytic astrocytoma in the posterior cranial fossa

A

Medulloblastoma
Midline 4th ventricular mass in 1st decade of life
Hyperattenuating (CT)
diffusion restricting (MR)

PCA -
Cystic cerebellar mass with enhancing mural nodule
Enlarged optic nerve/chiasm/tract with variable enhancement

Hypo/Isodense. Ca2+ often

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31
Q

Distinguishing features if ADEM?

A

Post viral. Young adults or children

Demyelination with bilateral asymmetrical T2/FLAIR hyperintensities (confluent or punctuate).

Thalamic involvement and cranial nerves (not typcial MS)

Dramatic response with steorids

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32
Q

What is Hurst disease?

A

Fulminant haemorrhagic version of ADEM

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33
Q

Rapidly progressive ataxia, ophthalmoplegia, dystonia, Lactic acidosis.
High T2 putamen, caudate periaqueductal grey matter?

A

Leigh Disease

Symmetric “speckled” lesions in basal ganglia (BG)

**L for Lactic acidosis **

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34
Q

What is a Pott puffy tumour?

A

during acute sinusitis, bony erosion and subperiosteal abscess formation. Causes forehead swelling and can have intracranial extension.

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35
Q

Chondrocalcinosis, premature OA changes and dark pigmentation in skin, particularly the ears?

A

Alkaptonuria / oschronosis

Nb Can be diagnosed in infancy - nappies stained black from urine.

AR condition. Excessive levels of homogentisic acid (HGA), affinity to accumulate in connective tissues.

Calcification and destruction intervertebral discs.

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36
Q

History of epilepsy, young, temporal lobe lesion, multi-cystic lesion?

A

Dysembryoplastic neuroepithelia tumours (DNET)

**Nb - Multiple cysts in the lesion ‘bubbly’ **.

Lack of oedema or enhancement is typical

DDX for a temporal lobe lesion and epilepsy -
—Ganglioglioma (not bubbly, can be cystic and usually enhance)

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37
Q

Bilateral painless parotid swellings with mixed cystic and solid lesions on US. Main DDX?

A

Sjorgens
HIV
Sarcoidosis (rare)
Mumps

Differentiate -
–HIV typically has cervical lymphadenopathy, not painful amd NO calcifcations.

–Sjogren’s Middle-aged female demographic &/or intraglandular calcifications

— mumps are painful

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38
Q

Baby, developmental delay, normal head size but diffuse white matter abnormalities with a stripey ‘trigoid’ appearance

A

Metachromatic Leukodystrophy

**Normal head size **

Most common leukodystrophy. Trigoid or butterfly appearance.

Spares subcortical u fibres

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39
Q

Macrocephaly in child less than 12 months, diffuse bilateral WM changes and subcortical u fibres, elevated NAA peak levels?

A

Canavans disease

Macrocephaly with diffuse ↑ white matter (WM) T2 and DWI signal, and ↑ N-acetyl aspartate (NAA) in MRS

**Subcortical u fibres **

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40
Q

Macrocephaly in child less than 12 months , frontal white matter Chages?

A

Alexanders disease

Symmetric, ↑ T2-signal bifrontal white matter (WM), spare u fibres

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41
Q

5-10 year old, WM changes that in the occipital lobes and splenium of CC, that progress frontal. Elevated serum VLCFA ?

A

Adrenoleukodystrophy

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42
Q

Baby under 6 months old, RUQ mass, cardiomegaly ?

A

Haemangioendothelioma (aka Infantile hepatic haemangiomas (IHH))

**Fine calcifications. **

Often found on foetal antenatal scans!!!

Can be solitary or multiple

Nb - behave like giant haemangioma on imaging -
Heterogenous on US, peripheral enhancement with gradual filling in on CT, flow voids on MRI.

can cause significant R-L heart shunting.

AFP + Endothelial growth factor is elevated.

Thrombocytopenia due to platelet trapping

Nb - not like adult epitheliod haemangioendothelioma

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43
Q

Painless abdominal mass, 6 year old child. US - large well defined solid heterogenous liver mass with raised AFP?

A

Hepatoblastoma

Coarse calcification

Precocious puberty is highly specific but rare.

Most common malignant hepatic tumour of childhood.
Young adolescents = HCC

Associated with* Beckwith Wiedemann syndrome. *

Can invade the vessels

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44
Q

Large abdominal mass in neonate, with respiratory compromise. Multicystic and enhancing septations.

A

Mesenchymal hamartoma

Benign tumour. Sheer size can cause diaphragms splinting.

No Ca2+ or haemmorhage.

May grow rapidly, leading to compartment syndrome, respiratory distress, rarely heart failure

AFP may be moderately elevated

Imaging: Multiloculated large cystic mass in 70%;

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45
Q

What is the best test to diagnosis Meckel diverticulum?

A

Technetium - Pertechnetate studies

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46
Q

Syndrome linked with Chromophobic RCC?

A

Birt-Hogg-Dube

Also pulmonary cysts (increased risk of spontaneous pneumothorax) and fibrofolliculomas (benign fibrous tumours related to the hair follicles).

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47
Q

Syndrome’s linked with Wilms tumours?

A

**Beckwith-Wiedmnann (3 montly US for screening) **
DRASH
WAGR

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48
Q

Neonatal jaundice, normal US and 6 and 24 hours HIDA scan show no excretion?

A

Biliary atresia

US - sometimes shows echogenic ‘triangular cord’ structure in the porta hepatis which is pathogonomic

DDx - Neonatal hepatitis - 6 or 24 hours HIDA will show bowel uptake

Nb
Hepatic activity after 5 minutes and no bowel activity after 6 and 24 hours

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49
Q

Main causes for Neonatal distal intestinal obstruction with microcolon?

A

A. Meconium Ileus

Meconium impacted at the terminal ileum. Linked to CF.
On WSCE - outlines meconium pellets obstructing terminal ileum.

B. Ileal atresia
On WSCE - Blind end of contrast column in ileum = Ileal atresia

C. Total colonic Hirschsprung disease

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50
Q

Main causes of neonatal distal intestinal obstruction with small distal /left colon?

A

A. Meconium plug syndrome (neonatal small left colon)

Nonpathological transient functional obstruction at the proximal descending colon**.

+/- if water souble contrast isnt therapeutic then - Rectal biopsy to exclude long segment HD

B. Colonic atresia
WSCE - blind ending small caliber distal colon (can be more proximal)

C. Hirschsprung’s disease
WSCE - Classically short rectosigmoid segment = small calibre rectum and dilated sigmoid (rectosigmoid ration <1).
Long segment - Transition zone from the small distal colon to the dilated proximal colon
**suction biopsy below this transition point.

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51
Q

Older child with distal intestinal obstruction or right iliac fossa pain/mass and normal appendix. Bilateral upper zone bronchiectasis ?

A

Distal intestinal obstruction syndrome

Secondary to CF
Older child meconium ileus equivalent

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52
Q

3 year old RUQ mass, hilar mass displacing the pancreatic head and causing intrahepatic duct dilatation. CT shows retroperitoneal lymphadenopathy?

A

Rhabdomyosarcoma

Most commonly arise form the CBD.
Expansile intraductal biliary mass in young child
Dilated intrahepatic bile ducts caused by downstream biliary obstruction

DDx - Choledochal cyst

53
Q

Lobular liver mass in young patient with a central fibrous scar that doesn’t enhance?

A

Fibrolamellar carcinoma

Central scar (low T1 and T2 as fibrous) that doesn’t enhance or Ca2+ in scar help differentiate from FNH

54
Q

Sacular dilatation of the proximal large intrahepatic biliary ducts, alongside focal strictures (beaded apperance) ?

A

Caroli Disease (Type V choledochal cyst)

AR disease linked with medullary sponge kidney, ARPKD and ADPKD and significant risk of cholangiocarcinoma

55
Q

Port wine stain, recurrent seizures, Gyral cortical calcification and leptomeningeal enhancement ?

A

Sturge-Weber syndrome

Cerebral lobar atrophy with cortical/subcortical Ca⁺⁺ leptomeningeal enhancement
enlarged ipsilateral choroid plexus

56
Q

What is the most common type of tracheoesophageal fistula ?

A

Type C

Type A - No/Absent TOF, just oesophageal atresia (9%)

Type B - oesophageal atresia with a fistula between the proximal pouch and the trachea

**Type C - oesophageal atresia with a fistula from the trachea or the main bronchus (Level of carina) to the distal oesophageal segment (80%)
**
Type D is oesophageal atresia with both proximal and distal fistulas

Type E or H - Isolated TOF with no oesophageal atresia.
Present later in life with recurrent URTIs

C and D: Gas in stomach & bowel

A and B: Gasless stomach & bowel

57
Q

Most specific sign on upper GI study of pyloric stenosis?

A

String sign

Narrow and elongated pyloric channel

58
Q

US measurements to Dx pyloric stenosis ?

A

Single wall thickness - 3mm
Pyloric transverse diameter: > 13 mm
Pyloric length - > 15mm
Pyloric volume > 1.4cm3

59
Q

Key difference between gastroschisis and omphalocele?

A

Omphalocoele -
-midline with surrounding membrane.
-Associated with multiple chromosomal abnormalities.
-VSD, neural tube defects, beckwith-wiedmann syndrome.
-Umbilical cord inserts in the apex of the defect

Gastroschisis -
-Paraumbilical
-Always on right side, no membrane, rarely associated with anomalies.
-Associiated intestinal astresia
Maternal AFP is raised.

60
Q

Congenital heterotaxy disorder with large ASD ?

A

Left sided isomerism (Polysplenia)

Bilobed lungs
liver positioned centrally
interrupted IVC with azygous or hemiazygos continuation
truncated pancreas.

Mortality by age 5 is 75% (slighlty better prognosis that right/asplenia)

61
Q

What cardiac abnormality in right sided isomerism ?

A

TAPVR

Severe cyanotic disease at birth. Mortality rate 80% by age 1.

Asplenia
trilobed lungs
IVC on same side as aorta
horseshoe kidney
Liver normal position.

62
Q

Lower lobe predominant bronchiectasis ?

A

Primary ciliary dyskinesia

50% have kartageners syndrome - situs inversus, sinusitis and bronchiectasis.

63
Q

Overinflation of the apicoposterior segment of the left upper lobe with ‘gloved finger’ sign?

A

Bronchial atresia

Gloved finger - is mucal impaction (mucocele). Presents usually in adolescences

Ovoid/round or tubular branching mass (finger-in-glove sign) with hyperinflated lung distally

64
Q

Term baby via C-section in diabetic mother. Respiratory distress?

A

Transient tachypnoea of the new-born (TTN)

Nb - normal descend down the vaginal canal squeezes fluid out of the lungs.

CXR -
bilateral symmetrical air space opacifications (nodular),WITH air bronchograms fluid in fissures, pleural effusions. No cardiomegaly

Peaks in severity in 24hrs, resolved by 3 days.

65
Q

Pre-term neonate diffuse granular opacities on CXR. lung volume appears low and air bronchograms?

A

Surfactant deficient disease
(a.k.a. respiratory distress syndrome & hyaline membrane disease)

DDx
Group B Streptococcal Pneumonia as ‘diffuse granular’ opacities + PLEURAL EFFUSIONS

Can go on to develop Bronchopulmonary dysplasia
-Varying degrees of hazy opacities, cysts, hyperinflation, & coarse linear opacities diffusely in premature infant
- 28 days needing 02 /respiratory suppport

66
Q

Post-term perihilar streaky/rope like opacites and asymmetric hyperinflation?

A

Meconium aspiration syndrome

Coarse, rope-like perihilar opacities bilaterally + hyperinflation in term infant
Air bronchograms ABSENT

Can develop PTx, pneumomediastinum or PIE

67
Q

PIE v bronchopulmonary dysplasia

A

PIE -
-Usually occurs during first 10 days of life

BPD
occurs after the baby in question has received ventilation of >21% oxygen for longer than 28 days.

68
Q

3 month old infant progressive respiratory distress. CXR - Hyperinflated left upper lobe with mass effect.

A

Congenital lobar overinflation

Check-valve bronchial obstruction.

Neonate with hyperexpanded lucent pulmonary lobe
Especially left upper lobe

May be fluid-filled in 1st few hours after birth

LUL commonest. Then RML

69
Q

Large unilocular fluid density mass in the subcarinal region?

A

Bronchogenic cyst

Malformation of bronchial tree and lined with respiratory epithelium so hence produce fluid.
**Do not usually communicate with bronchial tree so don’t contain air. **

Middle Mediastinal or Central location with NO feeding vessels

If small asymptomatic/incidental

70
Q
A

Congenital pulmonary airway malformation

-multiple gas filled cystic mass in the lung,
-contralateral mediastinal shift.

Often seen antenatal scan but can present neonatal respiratory distress or milder cases in child with LRTI and decompensation.

Can be cystic and solid masses thus CTA is performed to rule out a feeding vessel (pulmonary sequestration)

**Not lobe predominant. **

Remember CLO upper lobe and sequestration left lower lobe

71
Q

Premature baby who is ventilated. Deteriorating oxygen saturation’s and CXR shows overinflation, bubly and linear lucenies radiating from the hilum?

A

Pulmonary Interstitial emphysema

Alveolar rupture from barotrauma. prevalent in low birth weight and hyaline membrane disease.

New, “bubbly,” cystic or linear lucencies within lung of intubated premature infant

Can cause acute life threatening PTX and pneumomediastinum
Treated with high frequency oscillation ventilation

72
Q

Which type of sequestration has its own pleural lining?

A

Extralobular

Nb- its less common (25%) vs intralobular (75%)

Extralobular have additional congenital abnormalities - Diaphragmantic hernia/VSD

Venous drainage is into the IVC - so can present with respiratory distress to shunting

Extralobular - own pleura and therefore collateral air drift via the pores of Kohn cannot occur, lack of aeration helps differentiate it from intralobar sequestration. CXR as an opacity adjacent to the heart, abutting the diaphragm.

Intralobular has pore of kohn so cysts with fluid level

73
Q

What is the venous drainage of an intralobular sequestration?

A

Pulmonary veins

Nb - Intralobular sequestration typically present as isolated anomaly in older children with recurrent LRTIs.

74
Q

Older child with recurrent infections. Hyperlucent lung field smaller than the right, Bronchiectasis on CT?

A

Swyer-James syndrome

75
Q

Which congenital abnormalities are duct dependent?

A

Hypoplastic left heart syndrome
Severe Coarctation
Interrupted arch
Pulmonary atresia
Severe Ebstein’s anomaly
TOF (if no VSD)

76
Q

5 Ts of neonatal cyanotic and plethoric congenital heart disease?

A

TGA
Tricuspid atresia
Truncus arteriosus
TAPVC
Tingle ie single right ventricle

77
Q

The aorta and pulmonary trunk both arise from the right ventricle. There is a VSD. The baby has rockerbottom feet and overlapping fingers.

A

Double outlet right ventricle

Tingle in the 5 Ts

Edwards (trisomy 18) and Patau (trisomy 13) syndrome patients can exhibit rockerbottom feet and overlapping fingers

78
Q

Most common cyanotic congenital heart disease present after 12 months?

A

TOF

Nb - Boot shaped heart as the RVH turns the cardiac apex upwards.
Can have normal CXR when infant
Pulmonary oligemia
Strong association with right sided aortic arch

RVOT commonly implicated cause is infundibular stenosis at Pulmonary valve

79
Q

Chest radiograph shows cardiomegaly, large right atrium, pulmonary venous congestion with interstitial fluid

A

Hypoplastic left heart syndrome / aorta atresia

associated with coarctactaion

early onset (days) of cyanosis and heart failure, leading to collapse and death in a few weeks of life.

80
Q

Egg shaped heart on CXR.

A

TGA

transposition can be ‘corrected’ where the ventricles are also switched such that the correct ventricle feeds the correct great vessel although the left and right sided circulations remain separate.

CXR features -
‘egg on end’ or ‘egg on string’ appearance.
absent pulmonary trunk, and an abnormal aortic contour.

81
Q

Feautres of tricuspid atresia?

A

Absent functional tricuspid valve (TV)
No communication from right atrium to right ventricle (RV)

82
Q

NEonate VSD, PDA and dilated right atrium and right ventricle. Absent aortic knuckle and no oesophageal impression?

A

Interruption of the aortic arch

83
Q

Which diaphragmatic hernia is most common?

A

Bochdalek - 80%

Big,
Back (posterior),
Bad - present as Baby, contain Bowel and can be Bad As associated with pulmonary hypolasia due to mass effect
LEFT

Morgagni

Anteromedial aspect. Omental fat

84
Q

Very large enhancing hemi thoracic mass in a 1-2 year old - 2 key differentials?

A

A. Pleuorpulmonary blastoma
-Large pulmonary cystic, solid, or mixed mass in child < 6 years old

B. Askin tumour/Ewing Sarcoma of Chest Wall
-Early teens with large unilateral intrathoracic mass
-Large unilateral lobular opacity; may occupy entire hemithorax, particularly in combination with pleural effusion
**–Key differentiation feature is rib destruction = Askin **

85
Q

What cardiac tumour is strongly linked to Tuberous sclerosis ?

A

Rhabdomyoma

86
Q

Teenage age, tremor, behaviour changes, slurred speech. Xray generalised osteoporosis, chondrocalcinosis of TFC, subarticualr cysts?

A

Wilsons disease

WW2
**MRI - T2 hyperintensities Putamen and thalami. **

WM atrophy.
NB can have T1 high T1 signal - Copper.

Not may basal ganglia disorder have T1 hyperintensities all involve hepatic failure = -acquired non-Wilsonian hepatocerebral degeneration
-Wilson disease (copper)
-hepatic encephalopathy

‘Face of the giant panda sign’ = midbrain, when the red nucleus and substantia nigra are surrounded by high T2 signal in the tegmentum

87
Q

Child with symptoms of night pain in long bone diaphysis with sclerotic lesion?

A

Osteoid osteoma or Osteoblastoma

Depends on size < 1.5cm is osteoid osteoma > 2cm is osteoblastoma.

Diaphysis and posterior elements of spine are classic locations.

Nidus isn’t typically apparent on Xray - normally just seen sclerotic solid periosteal recaption.

CT helps identify the radiolucent nidus.

NB - Look for aspirin = classically reliefs pain

88
Q

Child with incidental lucent lesion in the long bone, diameatphyseal, sclerotic margin, eccentric and elongated?

A

Fibrous cortical defect if <2 cm

Non-ossifying firboma if > 2cm

Common benign

89
Q

Child after FOOSH with ulnar shaft fracture and radial head dislocation?

A

Monteggia

90
Q

Child after FOOSH with dislocation of the distal radio-ulnar joint with radial fracture?

A

Galeazzi

91
Q

Teenager and black, with densely calcified soft tissue mass at a joint with no bone involvement ?

A

Tumoral calcinosis

92
Q

What are the radiologically features of Perthes?

A

Small femoral epiphysis
Sclerosis of the femoral epiphysis
Soft tissue line changes
Widening of the joint space (effusion)
Radiolucent crescent subchondral lucent line

93
Q

Adolescent with pain in his leg for weeks, lucent, lobulated lesion with thin sclerotic margins at the proximal tibia epiphyses?

A

Chondroblastoma

The oedema on MRI can be florid.

Epiphysis and epiphyseal equivalent (tarsals, carpals, patella)

94
Q

What are the radiologically appearances of bone infarction?

A

Xray - Lucent area with a sclerotic serpiginous border of granulation tissue.

MRI - Serpiginous border returns low signal on T1 and a ‘double-line’ sign on T2 (high signal of granulation tissue surrounded by low signal of sclerosis)

Causes - Sickle cell, excess corticosteroid , pancreatitis, caisson disease (the bends), Gaucher disease, leukaemia/lymphoma, trauma

95
Q

Knee pain for few months, Lucent lesion with thick sclerotic margin in the tibial metaphysis. Pyrexia ?

A

Brodie abscess

Smoldering osteomyelitis.
children arise in the metaphysis as growth plater acts as physical barrier
In very young children (<18 months) this isnt the case and can be epiphysis and metaphysis .

on xray a lucent channel extending towards the growth plate it pathognomonic.

96
Q

15-year-old with lucent lesion in femur, permeative, periosteal reaction and associated soft tissue component?

A

Ewings sarcoma

Permeative/moth eaten appearance
Often Lamellated periosteal reaction can be sunburst
Predilection for DIAPHYSIS of long bones

1/3 have metastatic disease at presentation

97
Q

Adolescent Painful leg, fever. Poorly defined aggressive lesion with sunburst periosteal reaction and fluffy cloud like osseous matrix?

A

Osteosarcoma

Predilections for proximal tibia or distal femur (around the knee) = METAPHYSIS

Staging need MRI of entire bone affected. Nuclear bone scan and a CT chest.

Parosteal - lower grade, older age (20-50s)
Telangiectatic - purely lytic, poor prognosis, fluid-fluid levels
Multicentric - Age 5-10mvery poor prognosis

98
Q

Lower urinary obstruction in baby boy, enlarged urinary bladder with keyhole sign ?

A

Posterior urethral valves

Bladder funnels into a dilated posterior urethra
Hydronephrosis
Micturating cystogram will show fusiform dilatation of posterior urethra and trabeculation of the ballder with reflux

99
Q

Differentiate between multicystic dysplastic kidney and hydronephrosis ?

A

Hydronephrosis apparent cysts on US communicate with each other
Hydro is the most common cause of neonatal abdominal mass

Multicystic dysplastic kideny
-normal renal architecture is entirely replaced by a non-functioning mass of cysts separated from one another by fibrous tissue.
- ‘Bunch of grape’

100
Q

Most common solid renal mass in neonate?

A

Mesoblastic nephroma

*Most common Soild neonatal renal mass**

US - large hypoechoic renal mass which cannot clearly be separated from the normal renal parenchyma. Get nephrectomy.
**Doesnt invade into the collecting system or veins **

Multicystic dysplastic kidney 2nd commnest SOLID .

Any renal mass commonest is hydronephrosis.

101
Q

How to differentiate between mesoblastic nephroma and wilms tumour?

A

Mesoblastic nephroma do not invade into the collecting system/pelvis or venous.

US appearances are similar. Solid mass, iso or slightly hyperechoic, cysts/necrotic areas.
Both need resected, Mesoblastic nephroma is benign.

102
Q

Small testicle, homogeneously hypoechoic and enlarged echogenic epididmyis?

A

Chronic torsion

NB - Symptoms weeks

103
Q

Heterogenous testis with increased peri-testicular flow but absent testicular flow?

A

Missed Torsion

Nb - Symptoms lasting longer than 24 hours

104
Q

Most common solid abdominal mass in infants?

A

Neuroblastoma
Second most common in children of all ages to Wilms

105
Q

Key features of neuroblastoma?

A

Peak age is 2

Most adrenal in location, 1/3 retroperitoneum and 1/5 posterior mediastinum.

Secrete catecholamines = hypertension ir VIP = watery diarrhoea
**ENCASES the IVC **

Crosses midline

**Ca2+ **

‘Dropping lily sign’ on urography - kidney displaced Downard’s

**Staging is performed with an 123I-MIBG scan **
Stage IVs (disease involving the skin, bone marrow and liver) which has the best prognosis.

106
Q

Key features of Wilms (nephroblastoma) tumour?

A

peak age 3-4

**Displaces +/- invades the IVC
**
less commonly crosses the midline or Ca2+

‘Claw sign’ - Rim of normal renal tissue extending around the mass.

10% tumour - Ca2+ ( curvillnear), vascular invasion, bilateral, lung mets

107
Q

Abdominal mass in 4 year old boy, US mass of cysts with thick fibrous capsule. Mass protudes into the renal pelvis but no solid components?

A

Multilocular cystic nephroma

*Protusion into the renal pelvis

two age peaks; one at around age 4 which affects mostly boys and one at around age 40 which affects mostly women ie
‘Michael jackson’ tumour - like young boys and older women

108
Q

Features of idiopathic scrotal odema syndrome?

A

Scrotal swelling +/- acute pain.
Scrotal wall oedema and hypervascularity on US
Managed conservatively with NSAIDS

109
Q

Early teens female, amenorrhoea, elevated AFP. Ovarian lesion on US than shows low signal on MRI (T1 and T2) ?

A

Leydig-Sertoli tumour

They produce excess testosterone/oestrogen hence amenorrhoea.
Solid fibrous tumours that can have cystic elements with haemorrhage or necrosis

110
Q

What is autosomal recessive polycystic disease linked with ?

A

**Hepatic fibrosis - disease severity has inversion correlation
Caroli disease
Pancreatic fibrosis
Portal hypertension

111
Q

Dense bones with short stature, acroosteolysis, increased angle of mandible

A

Pycnodysostosis

AR
widened hands and feet
Receding jaw and beaked faces -Obtuse mandibular angle
Osteosclerosis is dominant radiographic finding - Does not obliterate marrow space

DDx
Osteopetrosis - Whole medula of long bone sclertotic

112
Q

Shortening of long bones with widening of the metaphysis and diaphysis. Kyphosis and beaking of the anterior vertebral bodies.

A

Mucopolysaccharidosis

metacarpals that are short, broad, and constricted proximally
Marcocephaly
Dysplastic changes in the femoral head
Odontoid hypoplasia → atlantoaxial instability

**Murquio = Vertebral beaking is middle anterior **

113
Q

Periosteal reaction, coarse trabeculation, epiphysel plates are widened, fraying at the metaphysis side?

A

Rickets

114
Q

Squaring of the patella and widending of the intercondylar notch?

A

Haemophilia arthopathy

115
Q

Exopthalmos, Diabetes insipdis and destrutive bony lesions?

A

Hand-schuller- Christian disease
dissemianetd Langerhans cell histocytosis

116
Q

Fusion of C2/C3, short neck and low hariline?

A

Kippel-Feil syndrome

117
Q

hypoplastic aorta and pulmonary trunk, rigth atrial enlargement. neonatal and cyanotic

A

Ebsteins anomaly

118
Q

Associations with truncus arterious

A

Forked ribs, DiGeorge syndrome, a right sided arch, or an interrupted arch are all known associations.

119
Q

causes of lung mets in children?

A

Wilms
Ewings
Oestosarcoma
Rhabdomyosarcoma

120
Q
A

stills disease

121
Q

Bilateral cerebriform, diffuselt enlarged adrenal glands

A

Congenital adrenal hyperplasia (CAH)

122
Q

paediatric liver tumours and a raised AFP

A

see chart

123
Q

NAI fractures

A

Child less than 1 = Neuroimaging

interhemispheric SDH highest specificity for abuse of any intracranial injruy

124
Q

Neonatal lines and tubes

A

ET tube - T3 or 1-1.5cm above the carina

UVC - At/just above diaphragm, usually T8-T9. IVC-RA junction

UAC -
Ideal high line: Descending thoracic aorta (T6-T10)
Acceptable low line: Distal abdominal aorta (L3-L4)

Nb cant be sited T12- L3 as banches of aorta and can cause stenosis

125
Q

widespread pervivascualr calcifcation in child with chemo and radiothearpy for acute leukemia?

A

Mineralising angiopathy

126
Q

Schizencephaly

A

Open-lip schizencephaly may result in large cystic cavity often mistaken for cyst

Closed-lip schizencephaly may be very small & subtle, mimicking transmantle GM heterotopia

transmantle GM heterotopia
-Column of grey matter extending from the subependymal to the pial surface.

DDx
porencephalic cyst
-intraparenchymal CSF-filled cavity that communicates with the ventricle and subarachnoid space.
-lined with ** gliotic white matter **
- previous insult - fetal life or early infancy , Brain destruction during perinatal period

Neuroglial cyst
- doesnt communicated with the lateral ventricle
- Nonenhancing, CSF-like parenchymal cyst with minimal/no surrounding signal abnormality

127
Q

Features of moyamoya

A

Attenuated COW with multiple tiny basal ganglia (BG) flow voids on MR

Large vessel occlusion- supraclinoid ICA/COW; anterior > > posterior circulation

“Puff of smoke” (moyamoya in Japanese)
Cloud-like lenticulostriate and thalamostriate collaterals on angiography

128
Q

grading of VUR

A

Grade I: Reflux into distal ureters

Grade II: Reflux into collecting system (without calyceal dilatation/blunting)

Grade III: All of the above, plus mild dilatation of the pelvis and calices

Grade IV: All of the above, plus moderate dilatation (clubbing of calices)

Grade V: All of the above, plus severe tortuosity of the ureter

Grades IV and V
require surgery to avoid renal scarring, renal impairment and/or hypertension.